Real-world drug utilization and treatment patterns in patients with tenosynovial giant cell tumors in the USA.

Aim: Real-world treatment patterns in tenosynovial giant cell tumor (TGCT) patients remain unknown. Pexidartinib is the only US FDA-approved treatment for TGCT associated with severe morbidity or functional limitations and not amenable to improvement with surgery. Objective: To characterize drug utilization and treatment patterns in TGCT patients. Methods: In a retrospective observational study using IQVIA's linked prescription and medical claims databases (2018-2021), TGCT patients were stratified by their earliest systemic therapy claim (pexidartinib [N = 82] or non-FDA-approved systemic therapy [N = 263]). Results: TGCT patients treated with pexidartinib versus non-FDA-approved systemic therapies were predominantly female (61 vs 50.6%) and their median age was 47 and 54 years, respectively. Pexidartinib-treated patients had the highest 12-month probability of remaining on treatment (54%); 34.1% of pexidartinib users had dose reduction after their first claim. Conclusion: This study provides new insights into the unmet need, utilization and treatment patterns of systemic therapies for the treatment of TGCT patients.

Treatment patterns in patients with tenosynovial giant cell tumors in the USAThis database study is the first investigation of how drugs are used to treat patients with tenosynovial giant cell tumor (TGCT) in the real world. We researched adult TGCT patients from IQVIA's prescription and medical claims databases who started treatment with pexidartinib (N = 82) or other non-US FDA-approved systemic therapies (N = 263). The patients included in this analysis were mostly women (61.0 and 50.6%) and their median age was 47 and 54 years for pexidartinib and other non-FDA-approved systemic therapies, respectively. The patients treated with pexidartinib were most likely to remain on treatment (54.0%) at the end of the first year. Most patients (79.3%) started pexidartinib treatment at a total daily dose of 800 mg/day, as per the product label. Only 34.1% of patients had reduced medication dose during follow-up. Of note, this study found that TGCT patients were treated with other systemic therapies which remain unproven to be safe and effective in medical studies of TGCT. Given the unmet need, and with pexidartinib being the only approved systemic treatment in USA, there is an opportunity for the larger population of adult TGCT patients to benefit from its use. Further research is needed to identify barriers for access to pexidartinib and treatment of TGCT patients.

Modified posterior approach of the knee in patients with diffuse pigmented Villonodular synovitis: case series of a single Institution's experience.

BACKGROUND: Diffuse pigmented villonodular synovitis (DPVNS) is a challenging tumor-like disorder that mainly occurs in the anterior aspect of the knee joint. The growth may sometimes extend to the posterior knee joint. Surgical excision is the mainstream treatment for DPVNS, and the posterior approach of tumor excision is adopted when the dominant tumor shows posterior extension. However, the optimal surgical approach over the posterior knee remains unknown. METHODS: Patients with DPVNS of the knee joint who received the posterior approach of synovectomy from 1995 to 2019 were retrospectively reviewed to describe the modified separate posterior (SP) approaches, and evaluate the treatment outcomes in a case series of DPVNS knees. The results of the SP approach was also compared with those of traditional direct posterior (DP) approach. Postoperative functional outcomes were evaluated using the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) standardized questionnaire and clinician-completed Musculoskeletal Tumor Society (MSTS) functional rating system at outpatient department. RESULTS: A total of 20 DPVNS knees were included. Thirteen patients who received SP approaches were included in the SP group, while seven patients who received the DP approach were included in the DP group. The median follow-up times were 5.7 years (IQR, 2-8.8) in the SP group and 3 years (IQR, 2-5.3) in the DP group. Both groups showed satisfactory safety. The SP group presented higher postoperative mean WOMAC (91.23 ± 7.20) and mean MSTS (24.23 ± 2.68) than the DP group (mean WOMAC: 76.00 ± 16.57; mean MSTS: 22.43 ± 4.69). The Wilcoxon signed-rank test was use to compare preoperative and postoperative range of motion (ROM) for each group. The significant difference in SP group (p = 0.004) was found while p = 0.131 in DP group. CONCLUSIONS: The SP approach provides an effective approach with satisfactory outcomes for the surgical treatment of DPVNS knees.

Localized tenosynovial giant cell tumor: a rare case of snapping hip.

We report on a 40-year-old male with a 9-month-long history of snapping of his right hip caused by a previously undescribed etiology of internal extra-articular snapping hip, namely due to a localized tenosynovial giant cell tumor. Both dynamic ultrasound evaluation and MRI proved to be crucial in the diagnosis of this rare entity. Auto-provocation of the snapping showed an anterior hip mass moving posteriorly to the psoas tendon which elucidated the pain and clicking sensation. Subsequent MRI demonstrated a peripheral low-intensity rim due to hemosiderin deposition around the synovial mass which is indicative for pigmented villonodular tenosynovitis. Treatment consisted of arthroscopic shaver burr resection. Immediately postoperatively, the snapping sensation could not be provoked anymore by the patient. The purpose of reporting on this case report is to emphasize several successive learning points. First, dynamic ultrasound aids in diagnosis and differentiation of the types of snapping hip. Second, specific MRI features are suggestive of tenosynovial giant cell tumor, recognizing these traits may prevent delayed diagnosis and subsequent aggravated clinical course. Third, localized pigmented villonodular tenosynovitis around the hip may present as an internal extra-articular snapping hip and is of consideration in the differential diagnosis of recurrent snapping hip.

Tenosynovial giant cell tumor-diffuse type, treated with a novel colony-stimulating factor inhibitor, pexidartinib: initial experience with MRI findings in three patients.

Tenosynovial giant cell tumor-diffuse type (diffuse TSGCT) is a benign but locally aggressive proliferative disorder of the synovium. Treatment is usually surgical, although in cases of extensive disease complete synovectomy is not possible and local recurrence rates are high. Pexidartinib (trade name Turalio®), a colony-stimulating factor-1 (CSF-1) inhibitor, was shown in a recent phase III trial to effectively treat diffuse TSGCT and is FDA approved for the treatment of adult patients with symptomatic diffuse TSGCT associated with severe morbidity or functional limitations and not amenable to improvement with surgery. Pexidartinib is available only through a restricted program under a risk evaluation and mitigation strategy (REMS) because of the risk of hepatotoxicity. Magnetic resonance imaging (MRI) is the preferred imaging modality for the diagnosis and surveillance of TSGCT. Here we present three patients with diffuse TSGCT of the knee who underwent multiple MRIs over several years while on pexidartinib. We describe the disease burden and signal characteristics on MRI and correlate with the response reported in the patients' medical records. Given that the use of pexidartinib and other CSF inhibitors is likely to increase, musculoskeletal radiologists should be aware of this novel non-operative treatment and the MRI appearance of diffuse TSGCT during therapy.

Outcomes of arthroscopic management for pigmented villonodular synovitis of the hip.

INTRODUCTION: Early synovectomy is considered as the main treatment of pigmented villonodular synovitis (PVNS) of the hip in young patients with preserved cartilage. The purpose of the study is to evaluate outcomes of arthroscopic management for PVNS of the hip. METHODS: Patients who underwent primary hip arthroscopy for the treatment of histology-confirmed PVNS by the senior author between January 2012 and December 2016 were retrospectively reviewed. We excluded patients who had less than 1-year follow-up or had undergone primary surgeries with other surgeons and then received revision hip arthroscopic procedures by the senior author. The recurrence of PVNS and postoperative condition of affected hip were assessed by follow-up magnetic resonance imaging (MRI). Patient-reported outcomes of modified Harris hip score (mHHS) and 12-item International Hip Outcome Tool (iHOT-12) were collected at latest follow-up. RESULTS: Nine patients (2 males, 7 females) with a mean age of 24.3 ± 11.2 years (range 14-44 years) were included in this study. Localized PVNS was observed in four patients, and diffuse PVNS was observed in five patients. No patient presented with advanced osteochondral destruction. Five patients received single adjuvant radiosynoviorthesis. No patient had evidence of recurrence based on follow-up MRI. Patient-reported outcomes were obtained in eight patients at mean 55.8 ± 26.1 months (range 24-84 months) after the index surgery. The mean mHHS was 94.6 ± 4.9 (range 84.7-100) and the mean iHOT-12 was 93.3 ± 20.2 (range 50-120). No patient needed secondary surgery during the follow-up period. CONCLUSION: Arthroscopic subtotal synovectomy can offer favorable short to mid-term outcomes in the treatment of hip PVNS in case of no advanced osteochondral damage at presentation.

Tenosynovial giant cell tumor.

Tenosynovial Giant Cell Tumor (TGCT) is a group of typically benign lesions arising from the synovium of joints, bursae and tendon sheaths. Depending on their growth pattern and clinical course, they are divided into localized and diffuse types. It is predominantly caused by a mutation in the stromal cells of the synovial membrane leading to overexpression of the colony stimulating factor 1 that recruits CSF1R-expressing cells of the mononuclear phagocyte lineage into the tumor mass. The lesions contain mainly histiocyte-like and synovial cells accompanied by varying numbers of multinucleated giant cells, mononuclear cells, foam cells, inflammatory cells and hemosiderin deposits. The gold standard for detect- ing and monitoring the disease is MRI, where the characteristic hemosiderin accumulation can be best appreciated, but it is a histological examination that is most conclusive. The main treatment is surgical resection of all pathological tissue, but radio- and chemotherapy are also viable options for certain groups of patients.

Management of Pigmented Villonodular Synovitis (PVNS): an Orthopedic Surgeon's Perspective.

PURPOSE OF REVIEW: Pigmented villonodular synovitis (PVNS) or tenosynovial giant cell tumor (TGCT) encompasses a wide spectrum of disease and is divided into localized and diffuse variants. Surgical resection remains the principal treatment for nearly all localized type disease and most diffuse type. Recent mechanistic understanding of the disease led to drug discovery that has opened new avenues for patients with recalcitrant disease. In this manuscript, we review the current treatment options for TGCT, presenting outcomes from traditional surgical approaches as well as those from nonsurgical approaches. RECENT FINDINGS: Arthroscopic and/or open surgery remains the mainstay of treatment for TGCT for the vast majority of patients. While radiosynoviorthesis and external beam radiation have been used for recalcitrant disease, recent understanding of the colony stimulating factor 1 receptor (CSF1R) pathway and its paracrine and autocrine role in TGCT has led to the development of targeted inhibitors. Their optimal role and efficacy are unclear due to limited number of high-quality studies and contradictory results; however, recent and ongoing studies suggest there may be a role for their use, especially in diffuse and/or refractory disease. Surgery remains the most common treatment for TGCT, however, there may be an increasing role for adjuvant therapies, including the new targeted agents. Weighing the side effects of these treatments against the symptomatic benefit on a patient-by-patient basis in this benign disease remains critical.

Pigmented villous nodular synovitis mimicking metastases on 18F-FDG PET/CT in a patient with rectal mucosal melanoma: a case report.

BACKGROUND: Mucosal melanomas are rare and have a high potential for metastasizing. Surgical resection is the treatment of choice for single distant metastases. Malignant melanoma usually shows the highest uptake of fluorine-18 fluorodeoxyglucose (18F-FDG). 18F- FDG positron emission tomography /computed tomography (PET/CT) is usually used for melanoma staging. An extensive literature review revealed only 4 published case reports and an original paper involving 8 cases (12 cases in total) of patients with skin melanomas in whom pigmented villous nodular synovitis (PVNS) mimicked metastatic melanoma, however, none of the melanomas reported were of rectal mucosal origin. CASE PRESENTATION: A 60-year-old woman presented with recent diagnosis of rectal mucosal melanoma, two additional 18F-FDG-avid lesions in the left ankle and left foot were detected on 18F-FDG PET/CT. Metastases were initially suspected; however, the final diagnosis was PVNS. CONCLUSIONS: This is the first report of PVNS mimicking metastases on 18F-FDG PET/CT in a patient with rectal mucosal melanoma. Although high 18F-FDG-avid lesions in patients with rectal mucosal melanoma are highly suspected to be metastasis and warrant an meticulous examination, the present case is a reminder that in such patients, not all lesions with high 18F-FDG uptake, especially those near a joint, are metastases and that more extensive resection is unnecessary.

The surgical outcome and recurrence rate of tenosynovial giant cell tumor in the elbow: a literature review.

BACKGROUND: Tenosynovial giant cell tumor (TSGCT) is a rare proliferative disorder of the synovium. Because of its aggressive nature and recurrence potential, treatment of TSGCT involves surgical resection with or without synovectomy. There is currently a paucity of literature describing the surgical management of TSGCT at the elbow. The aim of this study was to evaluate clinical outcomes and recurrence rates following open and arthroscopic excision of TSGCT in the elbow. METHODS: Electronic databases were searched for relevant articles relating to surgical management of TSGCT of the elbow. We included all patients who received surgical treatment for TSGCT, with no age limitations. We excluded any nonsurgical treatment studies. Seventy-seven articles were identified for screening, and a total of 27 patients from 24 studies were included for the review. RESULTS: The patients' mean (standard deviation [SD]) age was 40.3 (21.7) years, and the most common presenting symptoms included pain (18/27, 66.7%), swelling (19/27, 70.4%), and decreased range of motion of the elbow (9/27, 33.3%). The majority of patients underwent open excision with or without synovectomy (23/27, 85.1%). Of those undergoing open procedures, 16 (16/23,69.6%) had diffuse TSGCT and 14 (14/23, 60.9%) remained symptom free for a mean (SD) follow-up of 38.9 (25.4) months. Four patients (4/27, 14.8%) were treated arthroscopically, all of whom had diffuse disease. CONCLUSIONS: Our review found that open synovectomy appears to be an effective treatment for both localized and diffuse TSGCT in the elbow, and arthroscopic synovectomy is emerging as a method of surgical management for diffuse TSGCT. However, because of the limited number of patients undergoing surgery for TSGCT, further studies are needed to make a definite conclusion.

Modified Lemaire extra-articular stabilisation of the knee for the treatment of anterolateral instability combined with diffuse pigmented villonodular synovitis: a case report.

BACKGROUND: Diffuse pigmented villonodular synovitis (PVNS) of the knee is a rare proliferative joint disease associated with high recurrence rates following surgical treatment. Intra-articular joint instability in conjunction with PVNS implies complex reconstructive strategies due to the destructive nature of the disease. CASE PRESENTATION: Here, we present the case of a young patient with refractory PVNS and a chronic ipsilateral anterior cruciate ligament (ACL) rupture. Clinically, the patient presented with a grade 3 pivot shift phenomenon, indicating anterolateral rotational instability. Usually, PVNS implies a contraindication for ACL reconstruction due to the degenerative and pro-inflammatory joint microenvironment that is induced and maintained by PVNS. Therefore, we have performed a modified Lemaire extra-articular stabilization resulting in significant clinical improvement and subjective joint stability. In the latest follow-up examination at 12 months, the patient reported subjective joint stability and no swelling. In the clinical examination, the patient showed dynamic joint stability during walking. Additionally, the patient presented with grade 0 in pivot-shifting compared to the contralateral knee. The Lachman test exhibited no increased side-to-side difference and a firm endpoint. CONCLUSIONS: Extra-articular anterolateral stabilisation of the knee in patients having anterolateral knee instability combined with PVNS is a safe and efficient surgical treatment option yielding significant clinical improvement as well as subjective joint stability.

Distinct extra-articular invasion patterns of diffuse pigmented villonodular synovitis/tenosynovial giant cell tumor in the knee joints.

PURPOSE: Pigmented villonodular synovitis (PVNS)/tenosynovial giant cell tumor (TGCT) is a benign, proliferative lesion of the synovium, the bursa, and the tendon sheath. Little is known about the anatomical distribution pattern of diffuse extra-articular PVNS/TGCT around the knee joint. In this retrospective study, anatomical distribution of PVNS/TGCT using magnetic resonance imaging (MRI) and arthroscopy was analyzed. METHODS: This study was designed as a retrospective, observational cross-sectional study based on MRI and arthroscopy. Twenty-four PVNS/TGCT patients (24 knees) who underwent arthroscopic or posterior open surgery between 2009 and 2016 were enrolled. Of these, eight intra-articular and 16 diffuse extra-articular PVNS/TGCT of the knee were classified. The anatomical locations of the PVNS/TGCT masses were determined with a newly devised mapping scheme. Analysis was performed on the prevalence of each compartment and agreement rates between each compartment. RESULTS: The point prevalence of intra-articular posterior compartment was higher in diffuse extra-articular PVNS/TGCT group compared with intra-articular PVNS/TGCT group. The point prevalence of diffuse PVNS/TGCT was most prevalent in the extra-articular posterolateral compartment (12 out of 16 diffuse extra-articular PVNS/TGCT patients, 75%) and second most common in the below to joint capsule compartment (11 out of 16, 68.8%). The agreement rate was the highest between intra-articular posterolateral and extra-articular posterolateral compartments (75%). CONCLUSION: Extra-articular invasion of diffuse PVNS/TGCT occurred in specific patterns in the knee joint. Extra-articular lesions were always accompanied by lesions in intra-articular compartments. In particular, lesions in the intra-articular posterior compartments were observed in all of the diffuse extra-articular PVNS/TGCT patients. The point prevalence of diffuse extra-articular PVNS/TGCT for each compartment was the highest [12 out of 16 (75%)] in extra-articular posterolateral compartment. In contrast, invasion to the extra-articular posteromedial side was less frequent [5 out of 16 (31.3%)] than to the extra-articular posterolateral side. Knowing where the lesions frequently occur may provide important information for deciding the timing, method, and extent of surgery. LEVEL OF EVIDENCE: Level IV.

Pigmented villonodular synovitis.

Pigmented villonodular synovitis (PVNS) is a benign disease that rarely undergoes malignant transformation. There are two types of disease: localized (nodular tenosynovitis) and di used (pigmented villonodular synovitis/tenosynovitis) with intra- or extra-articular locations. The second one is limited to synovium of the burse (PVNB) or tendon sheath (PVNTS). The intraarticular lesions are usually located in the knee, hip, ankle and elbow joints. Histologically, PVNS is a tenosynovial giant cell tumor, characterized by proliferation of two types of mononuclear cells - predominantly small, histiocyte-like cells and larger cells with dense cytoplasm, reniform or lobulated nucleus, with accompanying multinucleated giant cells and macrophages overloaded with hemosiderin that give typical image on MRI - currently selected as a gold standard for its diagnosis. The classic X-ray and CT are non-specific but similar to ultrasound should be used to evaluate disease progression and treatment response if radiotherapeutic and pharmacological methods were selected for treatment. An open arthroscopic surgery could also be applied in selected cases.

Arthroscopically-assisted mini open partial synovectomy for the treatment of localized pigmented villonodular synovitis of the knee. A retrospective comparative study with long-term follow up.

PURPOSE: The purpose of this study was to present the long-term results of treatment of localized pigmented villonodular synovitis (LPVNS) comparing two operative procedures of excision of the lesion-the arthroscopic and the arthroscopically assisted mini-open. We hypothesized that the latter approach allowed for treatment of LPVNS with acceptable recurrence rates, complication rates and functional outcomes. METHODS: Between 1990 and 2006, 21 patients with LPVNS were treated with partial synovectomy through an arthroscopically-assisted mini open technique (group A), and 23 patients were treated with an arthroscopic excision of the lesion (group B). All patients were clinically examined at one, three, and 12 months post-operatively and graded by the Lysholm knee score and the Ogilvie-Harris score. RESULTS: The mean Lysholm score was improved from 58.7 ± 9 to 94.2 ± 7 for group A (p < 0.05) and from 57.4 ± 9.1 to 95.5 ± 8 for group B (p < 0.05). The mean Ogilvie-Harris score was improved from 7.2 ± 2 to 11.2 ± 0.9 for group A and from 7.1 ± 2 to 11.75 ± 0.5 for group B (p < 0.05). We encountered two cases of CRPS and one case of recurrence of the lesion in group A and no complications for group B. CONCLUSIONS: Arthroscopically-assisted mini open partial synovectomy is a safe alternative treatment, especially for surgeons without extended experience in arthroscopic techniques. The arthroscopic localization of the precise position of the lesion and its subsequent mini-open excision is a safe and effective technique with very low morbidity and recurrence rate and equivalent functional outcome to fully arthroscopic excision due to limited incision. LEVEL OF EVIDENCE: Retrospective comparative study, Level III.

PIGMENTED VILLONODULAR SYNOVITIS IN A RETICULATED GIRAFFE (GIRAFFA CAMELOPARDALIS).

: A 17-yr-old, female, captive-born reticulated giraffe ( Giraffa camelopardalis ) presented with acute-onset lameness of the right metacarpophalangeal (fetlock) joint. Despite multiple courses of treatment, the lameness and swelling progressively worsened over a 3.5-yr period, and the giraffe was euthanized. At necropsy, gross and microscopic changes in the right, front fetlock and associated flexor tendon sheath included villous synovial hyperplasia and the formation of discrete pigmented nodules within synovial membranes. Histologically, the nodules were composed of abundant, fibrous connective tissue with heavy macrophage infiltration, hemosiderin deposition, and distinctive, multinucleated cells that resembled osteoclasts. These findings were consistent with pigmented villonodular synovitis (PVNS), a rare condition affecting both humans and animals. Although the pathophysiology of PVNS is poorly understood, lesions exhibit features of both neoplastic and reactive inflammatory processes. This case report represents, to the authors' knowledge, the first description of PVNS in a nondomestic ungulate.

Arthroscopic treatment of intra-artricularly localised pigmented villonodular synovitis of the ankle: 4 cases with long-term follow-up.

BACKGROUND CONTEXT: Pigmented villonodular synovitis (PVNS) is a rare, locally aggressive benign proliferative pathology of synovial tissue. Lesions are classified regarding location as diffuse or localised which are same as histologically. Intra-articularly localised type is relatively rare, especially in the ankle joint. Because of the high recurrence rate and aggressive nature, localised lesions should be excised totally. PURPOSE: We performed a retrospective study of 4 patients with intra-articularly localised PVNS in the ankle joint who were treated by total arthroscopic excision and evaluated for functional results and recurrence rate. STUDY DESIGN: Case series. PATIENT SAMPLE: The mean age of the patients was 27 (17-46) years at the time of arthroscopic surgery. The mean follow-up time was 33 (24-48) months. METHODS: Functional evaluation according to the Musculoskeletal Tumour Society Score was performed, and patients were evaluated for recurrence by MR imaging. RESULTS: In all patients, functional results were excellent and there was no recurrence. CONCLUSIONS: This study demonstrates that the intra-articularly localised PVNS can be successfully treated with arthroscopic procedures without recurrence.

Pigmented villonodular synovitis: a crowdsourcing study of two hundred and seventy two patients.

PURPOSE: We aimed to ascertain the feasibility of crowdsourcing via Facebook for medical research purposes; by investigating surgical, oncological and functional outcome and quality-of-life (QOL) in patients with pigmented villonodular synovitis (PVNS) enrolled in a Facebook community (1112 members). METHODS: Patients completed online open surveys on demographics, surgery and clinical outcomes (group 1); and patient-reported outcome measures (PROMs) including knee-injury osteoarthritis outcome score (KOOS), hip-disability osteoarthritis outcome score (HOOS), Toronto extremity salvage score (TESS) and SF-36 (group 2). Mean follow-up was 70 months (12-374). Consistency checks were performed with Cohen's kappa statistic for intra-rater agreement. RESULTS: The first survey was completed by 272 patients (group 1) and 72 patients completed the second (group 2). In group 1, recurrence-rate was 58 % (69/118) after arthroscopic, 36 % (35/97) after open and 50 % (5/10) after combined synovectomy (p = 0.003). In group 2, recurrence-rate was 67 % (26/39) after arthroscopic and 51 % (17/33) after open synovectomy (p = 0.19). Recurrence-risk was increased for diffuse disease (OR = 16; 95%CI = 3.2-85; p < 0.001). Mean function and QOL did not differ after arthroscopic or open synovectomy: KOOS 49 vs. 58 (p = 0.24), HOOS 62 vs. 53 (p = 0.56), TESS 78 vs. 82 (p = 0.86), SF-36 61 vs. 66 (p = 0.41). Cohen's kappa statistic for intra-rater agreement was good to outstanding (κ = 0.68-0.95; p < 0.001). CONCLUSION: Local recurrence-risk was higher for diffuse-type disease and arthroscopic synovectomy. Functional outcome and QOL were comparable for both types of surgery. Gathering data via crowdsourcing seems a promising and innovative way of evaluating rare diseases including PVNS.

Technical tip: Dorsal transmetatarsal approach to intermetatarsal and deep plantar lesions.

UNLABELLED: Synovial and chondromatous lesions can extend dorsally, intermetatarsally and deep into the vault of the longitudinal and transverse plantar arches of the foot. The lesions may prove difficult to excise even with combined approaches. Two cases where a transmetatarsal approach by excision of the base of the third metatarsal enabled successful complete excision of the lesions are presented. LEVEL OF EVIDENCE: Level V, expert opinion.

Clinical and oncological outcomes after surgical excision of pigmented villonodular synovitis at the foot and ankle.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare benign neoplastic disease of the synovium of joints and tendon sheaths, which may be locally aggressive. It can be broadly classified into localised disease or more diffuse forms, with the latter more prone to recurrence after surgical excision. We describe our experience in the management of foot and ankle PVNS, focusing on the diffuse type. METHODS: Patients with PVNS were identified from a histology database from 2000 to 2010 at the University Hospitals of Leicester. The primary aim was to determine oncological outcomes and evaluate clinical outcomes with the Toronto Extremity Salvage Score (TESS) and the American Academy of Foot and Ankle Surgeons (AOFAS) scores. RESULTS: 30 patients, 16 males and 14 females with a mean age of 37±15 years, who underwent surgery, were identified. There were 22 nodular PVNS and 8 diffuse PVNS. The diffuse PVNS was more likely to be in the hindfoot (75%, 6/8), of which 50% (3/6) had osteoarthritis at presentation. The localised PVNS was mostly located in the forefoot (91%, 20/22). None of the localised PVNS had a recurrence. The surgical recurrence rate in this series was similar to the pooled recurrence rate from the literature [12.5% (1/8) compared to 12.2% (6/49)]. The mean TESS and AOFAS scores were 86 and 78, respectively. CONCLUSIONS: Diffuse PVNS is more likely to occur in the hindfoot and nodular PVNS is more common in the forefoot. Aggressive synovectomy alone is an effective treatment for diffuse PVNS, with good oncological and clinical outcomes.

Giant Cell Tumor in Tarsal Midfoot Bones: A Case Report.

Diffuse tenosynovial giant cell tumor (D-TGCT), previously known as pigmented villonodular synovitis (PVNS), is a benign, aggressive, and distracting proliferative synovial lesion. D-TGCT is commonly seen in large joints such as the knee and hip. We present the case of a 57-year-old female who initially presented with swelling on the left midfoot that increased over four years. Clinically, a ganglion was suspected on the left midfoot and an MRI showed a heterogeneous lobulated soft tissue mass on the superior aspect of the tarsal midfoot measuring 5.8 x 2.4 x 4.2 cm. The mass causing remodeling and bony erosion was more appreciated at the medial aspect of the talus bone and extended to the sinus tarsi and talocalcaneal joint space. Surgical excision of the mass was performed, and pathology reports found lobulated soft tissue lesions composed of mononuclear cells, multinucleated giant cells, sheets of foamy macrophages, inflammatory cells, and hemosiderin-laden macrophages. This case represents D-TGCT without atypia or malignancy based on the findings.

The CT knee arthrogram revisited.

The CT arthrogram is an underrated diagnostic study of the joint. Although MRI is considered superior to CT in joint imaging due to its higher resolution, CT arthrograms provide unique insights into the knee joint, with simultaneous dynamic assessment and an option for management in some conditions. In this pictorial essay, I will discuss the standard techniques and various pathologies affecting the knee joint and their CT arthrography appearance.